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Musculoskeletal Block > Connective tissue diseases and vasculitis > Flashcards

Connective tissue diseases and vasculitis Flashcards

1
Q

What are connective tissue diseases

A

Autoimmune rheumatic diseases

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2
Q

What is Systemic Lupus Erythematosus?

A

An inflammatory multisystem autoimmune disorder.

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3
Q

Who would most commonly present with SLE?

A

A female Afro-Caribbean of 20-40yrs

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4
Q

identify some factors related to the aetiology of SLE

A

genetics
oestrogen levels
immunological factors
environmental- UV light, smoking, Epstein-Barr Virus

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5
Q

Describe the pathogenesis of SLE

A

After apoptosis of cells cellular remnants remain called blebs which carry self antigens on their surface. These are normally cleared up but instead they travel to lymphoid tissue where they sensitise T-cells. This leads to the production of autoantibodies by B-cells, triggering of the complement system and abnormal cytokine production.

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6
Q

What characterises the pathology of SLE?

A

Deposition of IgG antibodies and complement proteins and influx of neutrophils + lymphocytes. Also vasculitis

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7
Q

There are many variant clinical manifestations of SLE. Joint involvement is the most common manifestation. What features may be shown

A

synovitis and joint tenderness 2+ joints
myalgia
Jaccoud’s arthropathy

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8
Q

How does SLE manifest in the skin?

A 
characteristic butterfly rash erythema on face
photosensitivity
purpura
urticaria
alopecia
Raynaud's phenomenon
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9
Q

As well as resulting in symptoms related to the joints and skin, what other ways may SLE present?

A 
interstitial lung disease, pleural effusions
pericarditis, endocarditis
lupus nephritis
depression, delirium, Psychosis, 
Sjorgen's syndrome
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10
Q

Main investigations for SLE?

A

blood test- FBC- leucopenia/thrombocytopenia/lymphopenia
autoantibodies- ANA, anti-dsDNA, anti-Ro, anti-Sm, anti-La
complements C3 and C4

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11
Q

treatment of SLE?

A

avoid excessive sun exposure
NSAIDs
hydroxychloroquine
corticosteroids

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12
Q

What is Primary Sjorgen’s syndrome? What are the main symptoms of Sjorgen’s?

A

The syndrome of dry eyes in the absence of RA and other autoimmune diseases
dry eyes, mouth, skin, vagina
fatigue
salivary and parotid gland enlargement

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13
Q

Which auto-antibodies are raised in Sjorgen’s?

A

anti-Ro, anti-La

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14
Q

Sjorgen’s increases risk of what serious condition?

A

Lymphoma

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15
Q

What are the two major characteristics of the pathology of Systemic Scleroderma (SSc)

A

Vasculopathy- widespread vascular damage

fibrosis- over lower dermis and internal organs (treatment symptom based or organ specific)

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16
Q

What is the main red flag feature of Systemic scleroderma?

A

Raynaud’s syndrome

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17
Q

What is the difference between LcSSc and DcSSc?

A

Limited and Diffuse cutaneous scleroderma differ in frequency 70%:30%, distribution (limited is restricted to hands, feet, face and forearms), and the clinical feature shown.

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18
Q

What are the main features of SSc?

A 
Raynaud's
Thickened skin- distribution varies- diffuse/limited?
Tight skin other face, small mouth and beaky nose
Telangiectasia
GI trouble
pulmonary hypertension
pulmonary fibrosis
myocardial fibrosis
scleroderma renal crisis
19
Q

Which are the two main autoantibodies shown in Scleroderma?

A

anti-centromeres

anti-topoisomerase

20
Q

How is Raynaud’s syndrome treated?

A

hand warmers
vasodilators- Calcium channel blockers e.g. nifedipine
PDE-5 inhibitors
prostacyclins

21
Q

What is the characteristic symptoms of the following conditions? Inflammatory myopathies, polymyalgia rheumatica and fibromyalgia

A

Myopathy- proximal muscle weakness
polymyalgia rheumatica- pain and stiffness
fibromyalgia- pain and fatigue

22
Q

The aetiology of inflammatory myopathies are _________. they are more common in _________. they have an associated risk of ___________. When the skin is involved it is called ___________.

A

unknown
women
malignancy
dermatomyositis

23
Q

What are the main clinical features of myalgia?

A

proximal muscle weakness
myalgia
difficulty with specific actions
insidious, over months or acute onset.

24
Q

What three signs distinguish polymyositis from dermatomyositis?

A

Gottron’s sign (purple discolouration of knuckles)
shawl sign
heliotrope rash (purple discolouration around eyes)

25
Q

How may inflammatory myopathies effect other organs?

A 
interstitial lung disease
dysphagia
myocarditis
polyarthritis
Raynaud's
26
Q

What may be identified in the blood tests of myositis patients? what other investigations would you do?

A

Raised CK, ANA, Myositis-specific antibodies, others
Electromyography
needle muscle biopsy
MRI

27
Q

Two main treatments of inflammatory myopathies?

A

corticosteroids and immunosuppression

28
Q

Which age group most commonly suffer from polymyalgia rheumatica?

A

almost exclusively over 50s

29
Q

What symptoms characterise polymyalgia rheumatica?

A

Pain and stiffness of shoulders and hip- worse in the morning
fever, fatigue weight loss

30
Q

What is giant cell arteritis also known as?

A

temporal arteritis

31
Q

What is giant cell arteritis?

A

It is an inflammatory granulomatous arteritis of large cerebral arteries which occurs in association with PMR

32
Q

What are the signs/symptoms of giant cell arteritis?

A 
severe headaches
tenderness and swelling of temporal arteries
jaw claudication
pain on the scalp
loss of sight in one eye
33
Q

What is the definitive test for PMR and GCA? What other test can be used?

A

temporal artery biopsy

temporal artery USS

34
Q

What are the starting daily doses of prednisolone for PMR and GCA?

A

PMR- 15mg
GCA- 40-60mg
afterwards is gradually reduced

35
Q

______________ is the most common cause of chronic musculoskeletal pain in women aged 20-50yrs

A

Fibromyalgia

36
Q

What are the major symptoms of fibromyalgia?

A

Chronic widespread pain
Fatigue- disrupted and unrefreshing sleep
tender points detectable on touch

37
Q

identify three pharmo treatments of FM and one non-pharmo treatment

A

Anti-depressants, gabapentin, pregabalin, analgesics

cognitive behavioural therapy

38
Q

Define vasculitis

A

Inflammation of blood vessel walls

39
Q

What are the two main features used in defining vasculitides?

A

size of the vessel- Large-vessel, medium-vessel, small-vessel
presence/absence of ANCA- Anti-neutrophil cytoplasmic antibodies

40
Q

What systemic symptoms are common to all vasculitides?

A

fever, malaise, fatigue, weight loss

41
Q

identify three similarities between Takuyasu’s arteritis and Giant cell arteritis

A

both large cell vasculitides
both effect aorta and its major branches
both show granulomatous inflammation

42
Q

How is vasculitis normally treated?

A

with corticosteroids and/or immunosuppressant drugs

43
Q

Give three examples of ANCA positive small cell vasculitides

A

granulomatosis with polyangiitis (GPA)
eosinophilic granulomatosis with polyangiitis (EGPA)
Microscopic polyangiitis (MPA)

44
Q

Give one example of an ANCA negative vasculitis?

A

Henoch-Schonlein Purpura (HSP)

Musculoskeletal Block (10 decks)

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